The Association Between Medicaid Expansion and Disparities in Vestibular Schwannoma Incidence.

OBJECTIVES: The effect of Medicaid expansion as a part of the Affordable Care Act on vestibular schwannoma (VS) incidence overall and in marginalized populations has not yet been elucidated. The goal of this study was to determine if Medicaid expansion was associated with increases in VS incidence overall, as well as in patients of non-white race or in counties of low socioeconomic status (SES). METHODS: We performed a difference-in-difference (DiD) analysis from January 1st 2010-December 31st 2017 utilizing the Surveillance, Epidemiology, and End Results (SEER) database. Our DiD method compared the change in VS rate between counties that did and did not expand Medicaid among patients of white and non-white race, in low and high SES counties, before and after expansion. RESULTS: The study included 17,312 cases across 1020 counties. Medicaid expansion was associated with a 15% increase (incidence rate ratio 95% CI: [11%, 19]) in VS incidence. White populations saw a 10% increase (CI: [1.06, 1.19]), Black populations saw a 20% increase (CI: [1.10, 1.29]), and patients of other races saw a 44% increase in incidence associated with expansion (CI: [1.21, 1.70]). Low SES counties saw an increase in incidence 1.12 times higher than that of high SES counties (CI:[1.04, 1.20]). CONCLUSION: Medicaid expansion was associated with increases in VS incidence across populations. Furthermore, this increase was more evident in disadvantaged populations, such as patients of non-white race and those from low SES counties. These findings emphasize the impact of Medicaid expansion on healthcare utilization for VS diagnosis. LEVEL OF EVIDENCE: Step/Level 3-Retrospective Cohort Study Laryngoscope, 2024.

Cochlear Implant Outcomes in Patients with Intralabyrinthine Schwannoma: A Scoping Review.

OBJECTIVE: To evaluate the literature and summarize cochlear implantation (CI) outcomes after intralabyrinthine schwannoma (ILS) excision and tumor observation with CI. DATA SOURCES: OVID Medline, Embase, Web of Science; conception to 2024. REVIEW METHODS: A literature review was performed using subject headings, MeSH terms, and keywords. Abstracts and full texts were reviewed by two independent reviewers and adjudicated by a third. Inclusion criteria included studies with ILS and CI with reported audiologic outcomes. Subjects were analyzed into two groups, ILS resection with CI and in situ ILS with CI. Patients with NF2 were included. The main outcome of interest was CI audiometric performance level, with secondary outcomes of CI user status and open-set speech attainment. RESULTS: There were 29 articles with a total of 93 patients who met inclusion criteria. The resection group had 17% low performers, 44% intermediate performers, and 38% high performers. The in situ group had 40% low performers, 32% intermediate performers, 27% high performers. The resection group had 69 patients with 97% maintaining user status and 92% with open-set speech recognition. The observation group had 24 patients, with 87% user rate and 86% achieving open-set speech recognition. There was a greater percentage of NF2 diagnosis seen in the in situ group. CONCLUSION: There is a paucity of literature on CI and ILS. Patients are managed with both resection of tumor and implantation in situ. Early data are limited, with improvement in hearing outcomes and high user rates in both populations. LEVEL OF EVIDENCE: N/A Laryngoscope, 2024.

The History of Superior Semicircular Canal Dehiscence: A Bibliometric Analysis.

OBJECTIVE: The treatment and understanding of superior semicircular canal dehiscence (SSCD) has seen significant developments over the past 25 years. Bibliographic analysis can provide insight into the evolution of research as well as highlight emerging areas. Reviewing a large volume of publications provides valuable insights into the citation patterns and collaborations of research groups. This study seeks to offer a comprehensive historical overview of SSCD and leading authors. DATABASE REVIEWED: Web of Science Core Collection. METHODS: A bibliometric analysis of the current literature on SSCD was conducted. A performance analysis and science mapping of the top 100 most cited articles was completed using a citation analysis. Two independent reviewers evaluated articles for relevance and adjugated by a third reviewer. Author and institution networks were examined. RESULTS: Seven hundred ninety-one articles on the topic of SSCD were identified. The top 100 articles spanned over 23 years from 1998 to 2019 and were published in 30 different journals. The top 100 articles were cited 8253 times in literature. The document contents revealed 233 keywords and 238 author keywords. The articles were authored by a total of 291 authors, with only 4 single-authored documents. CONCLUSIONS: SSCD has been highly researched in aspects of symptomatology, diagnosis, and treatment, as indicated by many highly cited articles that relate. However, the production of highly cited articles on SSCD displays a significant decrease after 2013, with only 17 of the top cited articles published since then.

Social Determinants of Health and Hearing Loss in U.S. Adults.

OBJECTIVES: Social determinants of health (SDH) are nonmedical, societal factors that influence health. There is limited information on the current relationship between SDH and hearing loss (HL) in the United States. This study aims to compare the odds of HL among US adults by race/ethnicity, education level, income-to-poverty level ratio, health insurance coverage, and health care access. STUDY DESIGN: Cross-sectional study. METHODS: The 2015-2020 National Health and Nutrition Examination Survey data were analyzed to compare odds ratios (ORs) for HL, defined as pure tone average over 25 dB HL in at least one ear, by SDH categories using sample weights. Adjusted ORs were calculated using logistic regression models controlling for sex, age, race/ethnicity, education level, income-to-federal-poverty level, health care insurance coverage and access, and loud noise, pesticide, and cigarette exposure. RESULTS: A total of 6028 participants were included. Non-Hispanic Black participants had half the odds of HL as Non-Hispanic White participants (OR 0.52, p < 0.05). Lower education level correlated with higher odds of HL: those without a high school diploma had double the odds of HL compared with college graduates or above (OR 2.05, 1.91, p < 0.05). The income-to-federal-poverty level ratio of 1.3 to less than 2 had higher odds of HL than the 4+ group (OR 1.45, p < 0.05). Use of multiple health care locations was associated with nearly three times the odds of HL than the group using one location (OR 2.87, p < 0.05). CONCLUSION: SDH are associated with HL. Further investigation is needed into the mechanism of disparities for targeted prevention and treatment for hearing care equity. LEVEL OF EVIDENCE: IV Laryngoscope, 134:2848-2856, 2024.

Review article: Otalgia as presenting symptom of myocardial infarction.

OBJECTIVE: There are many etiologies for otalgia, most of which are benign conditions. However, it can also be the initial symptom for life threatening emergencies such as a myocardial infarction (MI). This case report and review of literature describes diagnosis of MI with the initial primary complaint of unilateral otalgia. PATIENT: A 77-year-old female with intermittent left-sided otalgia with exertion for many years that recently worsened. In the clinic, she had a normal otologic exam and denied any other associated symptoms. The following day, her symptoms progressed to otalgia radiating down her arm and dyspnea on exertion. After progression of symptoms, a nuclear stress test was performed revealing a large and severe apical infarct. INTERVENTIONS: Diagnosis of referred otalgia from a cardiac source. She had subsequent medical management of cardiac risk factors and treatment of angina. RESULTS: Resolution of otalgia with medical management for coronary artery disease (CAD) after treatment of unstable angina. CONCLUSIONS: Otologists should be aware of the atypical presentations of angina because a missed diagnosis is potentially fatal. Otalgia, either unilateral or bilateral, may be the only presenting symptom of underlying cardiac ischemia and a high index of suspicion is needed for early diagnosis. Patients with otalgia related to exertion, no abnormal findings on otoscopic examinations, and significant risk factors for cardiac disease should undergo systematic evaluation including a cardiac rule out.

Medical School Factors Associated With Students Entering Otolaryngology.

OBJECTIVE: To determine medical school characteristics that are associated with medical students entering otolaryngology residency programs. STUDY DESIGN: Cross-sectional study. SETTING: Publically available data on otolaryngology residents and academic otolaryngology programs. METHODS: Publicly available websites were used to collect demographic and bibliometric characteristics for 1527 residents in Accreditation Council for Graduate Medical Education (ACGME)-accredited otolaryngology programs accounting for the 2017 to 2021 match periods. For each medical school, information on class size, number of otolaryngology faculty, presence of a home academic otolaryngology program, NIH research funding, presence of a student interest group, and top 10 ranking by Doximity or U.S. News and World Report (USNWR) were collected. Univariate and multivariate analyses were performed between the medical school factors and the percentage of each medical school class that matriculated into an otolaryngology residency program. RESULTS: On multivariate analysis, the following factors were found to be associated with a higher percentage of graduates entering an otolaryngology residency program: presence of a home academic otolaryngology program (standardized beta value (ß) = 0.397, p < 0.0001), a 2021 top 10 ENT ranking according to USNWR (ß = 0.206, p = 0.0028), and the average h-index of students from a medical school (ß = 0.327, p < 0.0001). CONCLUSIONS: After controlling for multiple factors including research productivity, we found that the presence of a home academic program and a top 10 ranking on USNWR were associated with an increasing percentage of medical school graduates entering otolaryngology. NIH funding and the number of otolaryngology faculty were not associated with more students matriculating into an otolaryngology residency program. These findings can help guide medical schools and otolaryngology programs to recruit students into the field.

Spinal Cord Injury at Birth, Expected Medical and Health Complexity in Chronic Injury Guided Anew by Activity-Based Restorative Therapy: Case Report.

As infancy is characterized by rapid physical growth and critical periods of development, disruptions due to illness or disease reveal vulnerability associated with this period. Spinal cord injury (SCI) has devastating consequences at any age, but its onset neonatally, at birth, or within the first year of life multiplies its impact. The immediate physical and physiological consequences are obvious and immense, but the effects on the typical trajectory of development are profound. Activity-based restorative therapies (ABRT) capitalize on activity-dependent plasticity of the neuromuscular system below the lesion and when provided to children with SCI aim to improve the child's neuromuscular capacity, health and quality of life. This is a report of an infant with a cervical SCI at birth resulting in paralysis of leg and trunk muscles and paresis of arm and hands who was enrolled in an ABRT program at 3 years of age. After 59 sessions of ABRT, the child demonstrated significant improvements in trunk control and arm function, as well as social and emotional development. Despite the chronicity of injury and low expectations for improvement with therapeutic interventions, ABRT had a positive impact on the child's physical capacity and provided benefits across multiple developmental domains.

Case Report: Capitalizing on Development and Activity-Dependent Plasticity, an Interaction With Pediatric-Onset Spinal Cord Injury.

Background: Spinal cord injury (SCI) in infancy halts typical development secondary to paralysis/paresis and the limited ability to engage with the environment. Traditional therapies further restrict a child via bracing, equipment, and medications. In contrast, activity-based restorative therapies (ABRT) promote activation of the neuromuscular system below the level of injury and affords a more typical sensorimotor experience. Case Description: A premature male infant exhibiting hypotonia, poor head control, and extremity weakness was diagnosed at age 5 months with a remote incomplete upper cervical SCI based on magnetic resonance imaging (MRI), presumed to have occurred perinatally. From 4 to 15 months of age, he received physical, occupational and speech therapies. Enrolled in an ABRT program at 15 months, he was unable to sit, pull-to-stand, stand, or walk and had upper extremity impairments. Results of the Bayley-III Scales of Infant and Toddler Development revealed gross and fine motor scores consistent with a 4-month-old. Methods: Activity-based restorative therapies was provided 5 day/week: 1.5 h of activity-based locomotor training and 1 h of activity-based occupational therapy. Results: Activity-based restorative therapies are reported for 177 sessions and are on-going. Improvements are noted in trunk control, standing, walking, grasp, in-hand manipulation, and associated kinematics. Bayley-III fine motor score improved to that of a 16-month-old and gross motor score to that of a 7-month-old. Discussion: While the two treatment periods (i.e., 4-15 months old and 15-24 months) were each ∼9 months, the child's accelerated progress toward typical development during the latter, ABRT period is noteworthy. In comparison to the period of traditional therapies in which paralysis was compounded by a restrictive environment and compensation, ABRT provided a potentially rich sensorimotor experience with an emphasis on active weight-bearing and proper kinematics to activate the neuromuscular system below the lesion in an age-appropriate, task-specific context of activities. Improved physical capacity enabled exploration more typically associated with development at this age expanding the positive impact to other developmental domains.

Developmental Anatomy of the Eustachian Tube: Implications for Balloon Dilation.

OBJECTIVE: To describe the developmental anatomy of the eustachian tube (ET) and its relationship to surrounding structures on computed tomography. STUDY DESIGN: Case series with chart review. SETTING: A tertiary care hospital. METHODS: ET anatomy was assessed with reformatted high-resolution computed tomography scans from 2010 to 2018. Scans (n = 78) were randomly selected from the following age groups: <4, 5 to 7, 8 to 18, and >18 years. The following were measured and compared between groups: ET length, angles, and relationship between its bony cartilaginous junction and the internal carotid artery and between its nasopharyngeal opening and the nasal floor. RESULTS: The distance between the bony cartilaginous junction and internal carotid artery decreased with age between the <4-year-olds (2.4 ± 0.6 mm) and the 5- to 7-year-olds (2.0 ± 0.3 mm, P = .001). The ET length increased among the <4-year-olds (32 mm), 5- to 7-year-olds (36 mm), and 8- to 18-year-olds (41 mm, P < .0001). The cartilaginous ET increased among the <4-year-olds (20 mm), 5- to 7-year-olds (25 mm), and 8- to 18-year-olds (28 mm, P < .0001). The ET horizontal angle increased among the <4-year-olds (17°), 5- to 7-year-olds (21°), and 8- to 18-year-olds (23°, P≤ .003), but the ET sagittal angle did not statistically change after 5 years of age. The height difference between the nasopharyngeal opening of the ET and the nasal floor increased among the <4-year-olds (4 mm), 5- to 7-year-olds (7 mm), and 8- to 18-year-olds (11 mm, P < .0001). CONCLUSION: The ET elongates with age, and its angles and relationship to the nasal floor increase. Although some parameters mature faster, more than half of the ET growth occurs by 8 years of age, and adult morphology is achieved by early adolescence.

Auditory Brainstem Implant in Adult Patient With Cochlear Ossification From Otosclerosis.

OBJECTIVE: The objective is to describe auditory brainstem implantation in a case of extensive cochlear otosclerosis. PATIENT, INTERVENTION, AND RESULTS: A case is presented of a 65-year-old male with bilateral cochlear otosclerosis and profound sensorineural hearing loss. Imaging studies showed distorted cochlear anatomy bilaterally and ossification of cochlear ducts. He underwent successful placement of an auditory brainstem implant using a retrosigmoid craniotomy approach. CONCLUSIONS: Extensive cochlear otosclerosis may distort cochlear anatomy such that cochlear implantation is expected to have a poor outcome. Auditory brainstem implantation may be an additional treatment option in these patients.

Bilateral Auditory Brainstem Implants in Patients With Neurofibromatosis 2.

OBJECTIVE: To discuss indications for bilateral auditory brainstem implants (ABIs), compare audiometric outcomes of unilateral vs bilateral ABIs, and determine if patients have improved outcomes with addition of a second-side implant. STUDY DESIGN: Retrospective review of 24 patients with neurofibromatosis 2 (NF2) who underwent sequential placement of ABIs from 1989 to 2019. SETTING: Tertiary referral center. METHODS: Charts were reviewed for indication for second-side surgery, use of implants, and audiometric outcomes. Implants placed in the past 30 years were included in the study. Northwestern University Children's Perception of Speech (NU-CHIPS) and/or City University of New York (CUNY) sentence scores were compared in unilateral and bilateral conditions. RESULTS: Indications for a second-side implant included first-side implants with severe nonauditory symptoms (11), marginal audiometric results (9), outdated technology (2), or deterioration of first side (2). Seven patients are bilateral users and 1 patient discontinued bilateral use after a year due to no significant improvement over unilateral use. One patient with initial bilateral use was lost to follow-up. Thirteen patients are unilateral users due to nonaudiometric side effects or poor audiometric outcomes with the first side. Two patients are complete nonusers. Seventy-five percent had improved audiometric outcomes after the second-side implant, and 20% had stable findings. CONCLUSIONS: Second-side ABIs should be consider in patients with poor performance from a first-side implant. Most patients demonstrate subjective improvement with the second ABI. More research is needed for better objective assessments of improvements.

Immune Response of Macrophage Population to Cochlear Implantation: Cochlea Immune Cells.

HYPOTHESIS: The presence and distribution of ionized calcium binding adaptor 1 and CD68 macrophages in the human cochlea is altered in cochlear implantation (CI) compared with the normative or nonimplanted cochlea. BACKGROUND: It has been hypothesized that CI induces an immunological response in macrophages leading to implant failure or reduced hearing. Macrophages are resident immune cells in human cochlea and have been shown to phagocytize implant material. In animal models, macrophage populations increase with surgical stress and with the introduction of a foreign body. However, the function and response of inner ear macrophages to CI are only beginning to be understood. This study seeks to investigate the inflammatory response to CI by comparing cochlear macrophages in implanted and nonimplanted human temporal bones. METHODS: Nineteen temporal bones from nine implanted ears, seven contralateral controls, and three normal control ears were evaluated for the presence and distribution of CD68 and Iba1 expressing positive macrophages. RESULTS: Three types of macrophage populations were detected 1) CD68 positive macrophages, 2) Iba1 positive macrophages, and 3) CD68 and Iba1 colocalizing macrophages. Macrophage distribution was ubiquitous: the stria vascularis, Rosenthal canal, and the mid-modiolus intermingled in the spiral ganglia. Iba1 and CD68 macrophages were found in the CI and non-CI contralateral and normal human cochlea. Most ionized calcium binding adaptor 1 expressing macrophages were ramified/amoeboid cells, while CD68 expressing macrophages were round shaped with foamy appearance in some areas. In the CI cochlea, both types of macrophages were detected in the fibrous sheath surrounding the CI path and within fibrotic areas within the scala tympani and the scala vestibuli in the case of CI translocation. In four cases, the density of macrophages was unchanged in the CI compared with the contralateral nonimplanted side, and in three cases, there was an increased number of macrophages in the implanted CI side compared with the nonimplanted side. CONCLUSION: Multiple populations of macrophages exist within the cochlea which are present at baseline and in response to trauma from CI. These results further support evidence for a macrophage response to cochlear implantation. Further studies are indicated to evaluate whether these macrophages have a beneficial, detrimental, or a mixed effect in CI patients.

Comparison of Transmastoid and Middle Fossa Approaches for Superior Canal Dehiscence Repair: A Multi-institutional Study.

OBJECTIVE: To compare outcomes for patients undergoing a transmastoid approach versus a middle fossa craniotomy approach with plugging and/or resurfacing for repair of superior semicircular canal dehiscence. Outcome measures include symptom resolution, hearing, operative time, hospital stay, complications, and revision rates. STUDY DESIGN: Multicenter retrospective comparative cohort study. SETTINGS: Three tertiary neurotology centers. SUBJECTS AND METHODS: All adult patients undergoing repair for superior canal dehiscence between 2006 and 2017 at 3 neurotology centers were included. Demographics and otologic history collected by chart review. Imaging, audiometric data, and vestibular evoked myogenic potential measurements were also collected for analysis. RESULTS: A total of 68 patients (74 ears) were included in the study. Twenty-one patients underwent middle fossa craniotomy repair (mean age, 47.9 years), and 47 underwent transmastoid repair (mean age, 48.0 years). There were no significant differences in age or sex distribution between the groups. The transmastoid group experienced a significantly shorter duration of hospitalization and lower recurrence rate as compared with the middle fossa craniotomy group (3.8% vs 33%). Both groups experienced improvement in noise-induced vertigo, autophony, pulsatile tinnitus, and nonspecific vertigo. There was no significant difference among symptom resolution between groups. Additionally, there was no significant difference in audiometric outcomes between the groups. CONCLUSION: Both the transmastoid approach and the middle fossa craniotomy approach for repair of superior canal dehiscence offer symptom resolution with minimal risk. The transmastoid approach was associated with shorter hospital stays and lower recurrence rate as compared with the middle fossa craniotomy approach.

A Histopathologic Comparison of Eustachian Tube Anatomy in Pediatric and Adult Temporal Bones.

HYPOTHESIS: In children, the distance between the carotid canal (CC) and Eustachian tube (ET) is not significantly narrower than the adult population. BACKGROUND: ET dysfunction treated with ET dilation is FDA approved for adults. Several studies describe the close relationship between the CC and the ET in adults, but the anatomy of the ET has not been well defined in children. This study seeks to investigate these relationships in the pediatric population. METHODS: Histologic sections from 23 temporal bones of pediatric patients ages 0 to 18 were reviewed by two independent observers. The distance between the CC and the cartilaginous Eustachian tube (CET), bony-cartilaginous junction (BCJ), and bony Eustachian tube (BET) were measured. Fifteen adult temporal bones were used as a control group. RESULTS: The distance to the CC was narrowest at the BET, and was actually higher in the pediatric population when compared to adults (0.5 mm and 0.2 mm, respectively, p = 0.06). The CC-CET distance was smaller in the pediatric group (2.3 mm vs 3.3 mm, p < 0.01). The bony-cartilaginous junction is often the region of most concern during dilation. There was no significant difference between the CC-BET distance in pediatric and adult groups (1.9 vs 2.3 mm, p = 0.20). CONCLUSIONS: CET-CC is smaller in the pediatric population, as expected due to smaller anatomic structures. However, the variable incline of the ET results in a CC-BCJ distance that is similar to the adult population. Although imaging studies are necessary.

Treatment of Ciprofloxacin-resistant Ear Infections.

OBJECTIVE: Ciprofloxacin resistance has been reported in 4.5% of patients with otorrhea and is increasing in prevalence. Due to ototoxicity, only fluoroquinolones are Food and Drug Administration approved for topical therapy in the middle ear. Furthermore, there is an assumption that antibiotic resistance is less relevant to topical therapy due to in vivo concentrations much higher than the minimum inhibitory concentration used to determine resistance. This study investigates ciprofloxacin-resistant infections and seeks to develop a better understanding of treatment options and outcomes. STUDY DESIGN: Retrospective review of 141 ciprofloxacin-resistant otologic infections. SETTING: Tertiary-care hospital. PATIENTS: Patients with culture-proven ciprofloxacin-resistant infections from 2008 to 2017. INTERVENTION(S): Antibiotic treatment with ciprofloxacin topical drops, ciprofloxacin plus oral antibiotics, and nonciprofloxacin therapy. MAIN OUTCOME MEASURE(S): Bacteriology for ciprofloxacin-resistant infections and treatment effectiveness of various therapies. RESULTS: Methicillin-resistant Staphylococcus aureus (33%), Corynebacterium striatum (19%), and non-Methicillin-resistant Staphylococcus aureus (11%) are the most frequent causes of ciprofloxacin-resistant infections. Topical ciprofloxacin monotherapy was successful in 2.7% of infections compared with a 64.7% success rate with the addition of an oral antibiotic (p < 0.001). Nonciprofloxacin drops are more effective with a 70% cure rate compared with the 2.7% of the ciprofloxacin drops p < 0.001. There was no difference in treatment efficacy when comparing nonciprofloxacin topical therapy (70% cure) to nonciprofloxacin topical therapy plus oral antibiotic (83% cure, p = 0.17). CONCLUSIONS: Using ciprofloxacin drops to treat ciprofloxacin-resistant bacteria is ineffective and patients do significantly better with alternative therapy. This finding supports the conclusion that high concentrations achieved in topical applications are not sufficient to overcome antibiotic resistance.

The Burden of Untreated HCV Infection in Hospitalized Inmates: a Hospital Utilization and Cost Analysis.

Hepatitis C virus (HCV) is highly prevalent in incarcerated populations. The high cost of HCV therapy places a major burden on correctional system healthcare budgets, but the burden of untreated HCV is not known. We investigated the economic impact of HCV through comparison of length of stay (LOS), frequency of 30-day readmission, and costs of hospitalizations in inmates with and without HCV using a 2004-2014 administrative claims database. Inmates with HCV had longer LOS, higher frequency of 30-day readmission, and increased cost of hospitalizations. Costs were higher in inmates with HCV even without advanced liver disease and in inmates with HIV/HCV compared to HCV alone. We conclude that although HCV treatment may not avert all of the observed increases in hospitalization, modest reductions in hospital utilization with HCV cure could help offset treatment costs. Policy discussions on HCV treatment in corrections should be informed by the costs of untreated HCV infection.

Otolaryngology and the Global Burden of Disease.

The Global Burden of Disease (GBD) project provides longitudinal analysis of the global burden of otolaryngologic diseases by measuring the all-cause mortality, years of life lost, the years of life lived with disability, and disability-adjusted life years. Hearing loss burden is assessed overall and as sequelae of other diseases, such as otitis media or meningitis. Using these measures, we can appreciate the high prevalence and disability related to hearing loss globally. Other otolaryngologic diseases that contribute to the GBD include otitis media, cleft lip and palate, head and neck cancer, facial trauma, and oral disorders.

CDH23 Related Hearing Loss: A New Genetic Risk Factor for Semicircular Canal Dehiscence?

OBJECTIVE: To investigate the prevalence and relative risk of semicircular canal dehiscence (SCD) in pediatric patients with CDH23 pathogenic variants (Usher syndrome or non-syndromic deafness) compared with age-matched controls. STUDY DESIGN: Retrospective cohort study. SETTING: Multi-institutional study. PATIENTS: Pediatric patients (ages 0-5 years) were compared based on the presence of biallelic pathogenic variants in CDH23 with pediatric controls who underwent computed tomography (CT) temporal bone scan for alternative purposes. INTERVENTIONS: Retrospective review of diagnostic high resolution CT temporal bone scans and magnetic resonance imaging (MRI) for evaluation of SCD. MAIN OUTCOME MEASURES: Superior and posterior semicircular canals were evaluated by a neuroradiologist for presence of SCD or abnormal development. RESULTS: Forty-two CT scans were reviewed for SCD. Eighty-six percent of the CDH23 variant group had abnormalities in at least one canal compared with only 12% in age-matched controls. In the CDH23 variant group there were four patients with superior SCD (57%, RR = 10.0) and three patients with posterior canal abnormalities (43%, RR = 7.5) compared with two, and two patients, respectively, in the control population. Four CDH23 variant children had bilateral abnormalities. One child had thinning or dehiscence in both the superior and posterior canals. Relative risk of SCD in children with CDH23 pathogenic variants is 7.5 (p < 0.001) compared with the pediatric control population. CONCLUSIONS: Children with a CDH23 pathogenic variants are at significantly increased risk of having SCD and this may be a contributing factor to the vestibular dysfunction in Usher syndrome type 1D patient population.

Nervus Intermedius Symptoms following Surgical or Radiation Therapy for Vestibular Schwannoma.

OBJECTIVES: This study compares the functional outcomes of nevus intermedius impairment following surgery, radiation, or observation for the treatment of vestibular schwannoma. STUDY DESIGN: Retrospective cohort study. SETTINGS: Tertiary care medical center. SUBJECTS AND METHODS: We retrospectively examined 141 charts of patients with a vestibular schwannoma seen in the Dartmouth-Hitchcock Acoustic Neuroma Clinic between 2012 and 2014. Seventy-one patients underwent intervention (including radiation) as their primary treatment, and 70 were treated with observation. As part of routine care, patients were interviewed at clinic visits and with a questionnaire assessing nervus intermedius impairment. RESULTS: At presentation, 25 patients (19%) reported nervus intermedius impairment. Most common disturbances were xerophthalmia (dry eyes, 13%) and dysgeusia (taste alteration, 7.6%). Postintervention, 35 patients (53%) had ≥1 symptoms of nervus intermedius dysfunction, which is increased in comparison with patients in the observation group (17 patients, 26.5%, P < .05). Twelve intervention patients had symptoms resolve postoperatively, making no long-term difference between the observation and intervention groups (P = .20). Motor function of the facial nerve postoperatively is correlated with nervus intermedius symptoms. Surgical approaches were compared with radiation therapy, and no significant difference in nervus intermedius outcomes was found. CONCLUSION: This study demonstrates the clinical importance of monitoring nervus intermedius symptoms, since a high percentage of all patients undergoing intervention will be symptomatic during management. Patients with motor dysfunction are at a higher risk of developing nervus intermedius sequelae and need close follow-up. Although impairment is common, many symptoms will improve over time with no long-term difference between intervention patients and those under observation.